Eosinophilia heralding the diagnosis of eosinophilic fasciitis (Shulman's disease)

Eosinophilic fasciitis or Shulman's disease is characterized, in its typical form, by palpable thickening of the skin and soft tissues, blood hypereosinophilia and fascia lesions. We hereby report a case of eosinophilic fasciitis in which hypereosinophilia preceded for several months the clinical signs of fasciitis. A 64-year-old woman, with a history of Little's syndrome with motor disability, was admitted in internal medicine for eosinophilia. For almost three months, no origin to the eosinophilia was found. The secondary onset of an edema and pain located on four limbs led to the diagnosis of eosinophilic fasciitis. Muscle magnetic resonance imaging was supportive and the muscle histological analysis confirmed the diagnosis of eosinophilic fasciitis. Treatment with steroids induced a rapid normalization of the eosinophilia and edema. In this case report, eosinophilia was preceding the clinical cutaneous signs that led to the diagnosis of eosinophilic fasciitis. It is likely to believe that myalgias, frequently found in the onset of eosinophilic fasciitis, may have been hidden by the history of infantile encephalopathy. The diagnosis of eosinophilic fasciitis must be kept in mind of physicians in the investigation of an eosinophilia, even though cutaneous signs are lacking.